RESUMO
Ectopic ACTH syndrome (EAS) remains one of the most demanding diagnostic and therapeutic challenges for endocrinologists. Thymic neuroendocrine tumors account for 5%-10% of all EAS cases. We report a unique case of a 31-year-old woman with severe EAS caused by primary metastatic combined large-cell neuroendocrine carcinoma and atypical carcinoid of the thymus. The patient presented with severe hypercortisolemia, which was successfully controlled with continuous etomidate infusion. Complex imaging initially failed to detect thymic lesion; however, it revealed a large, inhomogeneous, metabolically active left adrenal mass infiltrating the diaphragm, suspected of primary disease origin. The patient underwent unilateral adrenalectomy, which resulted in hypercortisolemia resolve. The pathology report showed an adenoma with adrenal infarction and necrosis. The thymic tumor was eventually revealed a few weeks later on follow-up imaging studies. Due to local invasion and rapid progression, only partial resection of the thymic tumor was possible, and the patient was started on radio- and chemotherapy.
Assuntos
Neoplasias das Glândulas Suprarrenais , Carcinoma Neuroendócrino , Síndrome de Cushing , Neoplasias do Timo , Humanos , Feminino , Adulto , Neoplasias do Timo/complicações , Neoplasias do Timo/patologia , Neoplasias do Timo/cirurgia , Síndrome de Cushing/etiologia , Síndrome de Cushing/patologia , Carcinoma Neuroendócrino/patologia , Carcinoma Neuroendócrino/secundário , Carcinoma Neuroendócrino/complicações , Carcinoma Neuroendócrino/cirurgia , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/patologia , Síndrome de ACTH Ectópico/diagnóstico , Síndrome de ACTH Ectópico/patologia , Síndrome de ACTH Ectópico/etiologia , Adrenalectomia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Primárias Múltiplas/complicaçõesRESUMO
BACKGROUND: Congenital adrenal hyperplasia (CAH) encompassed a bunch of autosomal recessive disorders characterized by impaired cortisol levels due to an enzymatic deficiency in steroid synthesis. In adult male patients with CAH, a frequent complication related to poor disease control is the development of ectopic adrenocortical tissue in the testes, named testicular adrenal rest tumors (TART). Conversely, ovarian adrenal rest tumors (OART) in females are extremely rare and adrenal rests in sites other than gonads are so uncommon to have been described only few times in literature. CASE PRESENTATION: We report a case of a male patient with untreated CAH and oncologic history of pleomorphic sarcoma who presented with massive bilateral adrenal enlargement and adrenal rest tumors in peri-lumbar and peri-cecal sites, which mimicked metastasis from sarcoma. CONCLUSIONS: The development of massive adrenal enlargement and ectopic adrenal rest tumors in sites other than gonads, even if very uncommon, should be suspected in patients with CAH and prolonged periods of undertreatment.
Assuntos
Hiperplasia Suprarrenal Congênita , Tumor de Resto Suprarrenal , Humanos , Hiperplasia Suprarrenal Congênita/complicações , Hiperplasia Suprarrenal Congênita/patologia , Hiperplasia Suprarrenal Congênita/diagnóstico , Masculino , Tumor de Resto Suprarrenal/patologia , Tumor de Resto Suprarrenal/diagnóstico , Tumor de Resto Suprarrenal/etiologia , Diagnóstico Diferencial , Sarcoma/diagnóstico , Sarcoma/patologia , Adulto , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/secundário , PrognósticoAssuntos
Neoplasias das Glândulas Suprarrenais , Lutécio , Compostos Radiofarmacêuticos , Idoso , Humanos , Masculino , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/radioterapia , Dipeptídeos/uso terapêutico , Compostos Heterocíclicos com 1 Anel/uso terapêutico , Lutécio/uso terapêutico , Antígeno Prostático Específico , Neoplasias da Próstata/radioterapia , Neoplasias da Próstata/patologia , Radioisótopos/uso terapêutico , Compostos Radiofarmacêuticos/uso terapêuticoRESUMO
Recently, the treatment landscape for metastatic pheochromocytomas and paragangliomas (MPPGL) has seen both progress and setbacks. We provide an up-to-date review of the multimodality management of MPPGL and discuss novel opportunities and current challenges in the treatment landscape. Given the unique clinical presentation of MPPGL, we discuss the management of hormone-related clinical sequelae and traditional modalities of therapy. Advances in the understanding of the molecular biology of these diverse tumors have enabled novel strategies such as augmenting DNA damage by targeted delivery of radionuclides such as 131I and 177Lu, abrogating tumor angiogenesis, hypoxia resistance, and DNA damage repair. Despite progress, we address the significant challenges still faced by patients and researchers engaged in efforts to improve outcomes in these rare cancers.
Assuntos
Neoplasias das Glândulas Suprarrenais , Paraganglioma , Feocromocitoma , Humanos , Feocromocitoma/terapia , Feocromocitoma/patologia , Feocromocitoma/secundário , Neoplasias das Glândulas Suprarrenais/terapia , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/patologia , Paraganglioma/terapia , Paraganglioma/patologia , Terapia Combinada , Gerenciamento ClínicoRESUMO
OBJECTIVES: Assess safety and efficacy of thermal ablation for adrenal metastases (AM) secondary to non-small cell lung cancer (NSCLC). MATERIALS AND METHODS: This retrospective study included patients with NSCLC AM treated with thermal ablation between 2/2010-11/2021. Local tumor progression free survival (LTPFS) and overall survival (OS) were calculated using Kaplan-Meier method. Adverse events were graded using Common Terminology Criteria for Adverse Events v5. RESULTS: Seven patients (mean age ± SD, 63.9 ± 12.5 years; 6 males) with seven AM were treated in eight sessions. Retreatment was performed in one patient with residual disease. Five sessions were with microwave ablation and 3 with radiofrequency ablation. Mean tumor size was 20.1 ± 7.0 mm. Median number of ablation probes used was 1 (range, 1-5), with a median of 3 activations (range, 1-3), and average ablation time of 14.4 ± 15.0 minutes. Response based on RECIST v 1.1 or PERCIST criteria revealed stable disease in 1 tumor, progression of disease in 3 tumors (one was re-ablated), and partial response in 3 tumors. Median LTPFS was not reached (NR) [95 % CI: 1- NR]. Median OS was 47.97 months (95 % CI: 18.63- NR). Intraprocedural hypertension (blood pressure ≥180 mmHg) occurred during 5/8 (62.5 %) sessions and intraoperative tachycardia occurred during 2/8 (25 %) sessions. Complications within one month of ablation occurred in 3/8 (37.5 %) sessions: grade 2 pneumothorax, grade 1 hematuria, and grade 2 adrenal insufficiency. CONCLUSIONS: In this small series, thermal ablation for NSCLC AM resulted in prolonged local control and OS with no major complications.
Assuntos
Neoplasias das Glândulas Suprarrenais , Carcinoma Pulmonar de Células não Pequenas , Neoplasias Pulmonares , Humanos , Masculino , Pessoa de Meia-Idade , Feminino , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/cirurgia , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Neoplasias Pulmonares/secundário , Estudos Retrospectivos , Idoso , Taxa de Sobrevida , Carcinoma Pulmonar de Células não Pequenas/patologia , Carcinoma Pulmonar de Células não Pequenas/cirurgia , Carcinoma Pulmonar de Células não Pequenas/secundário , Seguimentos , Prognóstico , Ablação por Radiofrequência/métodos , Ablação por Radiofrequência/efeitos adversos , Ablação por Cateter/métodos , Ablação por Cateter/efeitos adversosRESUMO
Pheochromocytomas are rare tumours originating in chromafï¬n cells, representing 0.1%-1% of all secondary hypertension cases. The majority are benign and unilateral, characterised by the production of catecholamines and other neuropeptides. Mainly located in the adrenal gland, they are more frequent between the third and fifth decades of life. Iodine-131 metaiodobenzylguanidine (131I-MIBG), a radiopharmaceutical agent used for scintigraphic localisation of pheochromocytomas, has been employed to treat malignant pheochromocytomas since 1983 in a few specialised centres around the world. We reviewed our clinical experience in one such case of a young lady who presented with history of abdominal pain, headache and lower back pain. On evaluation, ultrasonography revealed a right adrenal mass and elevated urine vanillylmandelic acid levels. Following surgical resection and histopathological confirmation of pheochromocytoma, MIBG scintigraphy revealed osseous metastases and hence, she underwent 131I-MIBG therapy.
Assuntos
3-Iodobenzilguanidina , Neoplasias das Glândulas Suprarrenais , Feocromocitoma , Compostos Radiofarmacêuticos , Humanos , 3-Iodobenzilguanidina/uso terapêutico , Feminino , Neoplasias das Glândulas Suprarrenais/secundário , Compostos Radiofarmacêuticos/uso terapêutico , Adulto , Intervalo Livre de Doença , Radioisótopos do Iodo/uso terapêutico , Neoplasias Ósseas/secundário , Neoplasias Ósseas/radioterapia , CintilografiaRESUMO
Metastatic pheochromocytomas and paragangliomas (PPGLs) are frequently associated with skeletal complications. Primary objective: to describe the frequency of adverse skeletal related events (SREs) in PPGL patients with bone metastases (BMs). Secondary objectives: to 1) identify predictive and prognostic factors for SREs and 2) obtain information on the effectiveness of bone resorption inhibitors in reducing SRE risk and improving outcomes in term of survival and SREs time onset. In this retrospective multicenter, multinational study, 294 PPGL patients were enrolled. SREs occurred in 90 patients (31 %). Fifty-five patients (19 %) had bone fractures, 47 (16 %) had spinal cord compression, and 11 (4 %) had hypercalcemia. Twenty-two patients (7 %) had more than one SRE. Sixty-four patients (22 %) underwent surgery, and 136 (46 %) underwent radiotherapy. SREs occurred a median of 4.4 months after diagnosis of BM (range, 0-246.6 months). Independent factors associated with reduced risk of SREs in multivariable analysis were I-131-MIBG radionuclide therapy (hazard ratio [HR], 0.536 [95 % CI, 0.309-0.932]; P = .027) and absence of liver metastases (HR, 0.638 [95 % CI, 0.410-0.992]; P = .046). The median overall survival duration was 5.3 year. In multivariable analysis, age younger than 48 years at PPGL diagnosis (HR, 0.558 [95 % CI, 0.3877-0.806]; P = .002), absence of liver metastases (HR, 0.618 [95 % CI, 0.396-0.965]; P = .034), treatment with bisphosphonates or denosumab (HR, 0.598 [95 % CI, 0.405-0.884]; P = .010), and MIBG radionuclide therapy (HR, 0.444 [95 % CI, 0.274-0.718]; P = .001) were associated with a reduced risk of death. SREs occur frequently and early in bone-metastatic PPGL patients but do not negatively impact survival. MIBG radionuclide therapy and treatment with bone resorption inhibitors are associated with favorable outcome.
Assuntos
Neoplasias das Glândulas Suprarrenais , Neoplasias Ósseas , Paraganglioma , Feocromocitoma , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Neoplasias Ósseas/secundário , Neoplasias Ósseas/complicações , Feocromocitoma/complicações , Feocromocitoma/patologia , Feocromocitoma/mortalidade , Estudos Retrospectivos , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/mortalidade , Neoplasias das Glândulas Suprarrenais/terapia , Adulto , Idoso , Paraganglioma/complicações , Paraganglioma/patologia , Paraganglioma/mortalidade , Adulto Jovem , Compressão da Medula Espinal/etiologia , Fraturas Ósseas/etiologia , Adolescente , Idoso de 80 Anos ou mais , Hipercalcemia/etiologia , Fatores de Risco , Conservadores da Densidade Óssea/uso terapêutico , PrognósticoAssuntos
Neoplasias das Glândulas Suprarrenais , Hemorragia , Melanoma , Neoplasias Cutâneas , Humanos , Melanoma/secundário , Hemorragia/etiologia , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias Cutâneas/secundário , Neoplasias Cutâneas/patologia , Masculino , Tomografia Computadorizada por Raios X , Pessoa de Meia-Idade , Doenças das Glândulas Suprarrenais/diagnóstico , Doenças das Glândulas Suprarrenais/diagnóstico por imagemRESUMO
ABSTRACT: An 84-year-old man with prostate adenocarcinoma underwent 68 Ga-PSMA PET/CT due to PSA recurrence. Foci of 68 Ga-PSMA uptake were observed in bilateral adrenal glands. Adrenal MRI showed metastasis only in the left adrenal gland. Metastatic 68 Ga-PSMA uptake was also observed in the mediastinum and bone. Enzalutamide treatment was started. Follow-up 68 Ga-PSMA PET/CT scan showed regression in both adrenal gland metastases and other metastases.
Assuntos
Adenocarcinoma , Neoplasias das Glândulas Suprarrenais , Isótopos de Gálio , Radioisótopos de Gálio , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Neoplasias da Próstata , Humanos , Masculino , Neoplasias da Próstata/diagnóstico por imagem , Neoplasias da Próstata/patologia , Idoso de 80 Anos ou mais , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/secundário , Adenocarcinoma/diagnóstico por imagem , Adenocarcinoma/secundário , Ácido Edético/análogos & derivados , OligopeptídeosRESUMO
PURPOSE: To study the prevalence of primary adrenal tumors and adrenal metastases in patients with neuroendocrine neoplasms (NENs) and describe these in detail. NENs can be further divided into neuroendocrine tumor (NET) and neuroendocrine carcinoma (NEC). METHODS: A review of medical files was conducted for all patients who underwent a 68Gallium-DOTATOC-PET/CT during 2010-2023 or adrenalectomy during 1999-2023 at the Karolinska University Hospital. RESULTS: In total, 68Gallium-DOTATOC-PET/CT was performed on 1750 individuals with NEN, among whom 12 (0.69%) had adrenal tumors. Of these, 9 (0.51%) were NEN metastases. Out of 1072 adrenalectomies, 4 (0.37%) showed evidence of NEN metastases. Thus, 16 patients with NEN exhibited adrenal tumors. The adrenal tumors were found on average 5 years after the NEN diagnosis and 19% of the adrenal tumors with simultaneous NEN were benign. Few had all adrenal hormones measured. None had an adrenal insufficiency nor an adrenal biopsy. Another synchronous metastasis was found in 69% at the time of the adrenal tumor discovery. During the median 2-year follow-up, 38% of the subjects had deceased (with the exclusion of individuals presenting supposedly benign adrenal tumors 31%) all due to tumor complications. A comparison between individuals identified through 68Gallium-DOTATOC-PET/CT and those who underwent adrenalectomy revealed a higher prevalence of NETs in the former group and NECs in the latter group. CONCLUSION: Adrenal primary tumors and adrenal metastases are infrequent occurrences in patients with NEN. Most cases involved the presence of NEN metastasis upon the initial discovery of adrenal tumors. The overall prognosis was found to be favorable.
Assuntos
Neoplasias das Glândulas Suprarrenais , Adrenalectomia , Tumores Neuroendócrinos , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Humanos , Neoplasias das Glândulas Suprarrenais/epidemiologia , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/secundário , Pessoa de Meia-Idade , Masculino , Feminino , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/epidemiologia , Idoso , Adulto , Estudos Retrospectivos , Prevalência , Carcinoma Neuroendócrino/patologia , Carcinoma Neuroendócrino/epidemiologia , Idoso de 80 Anos ou maisRESUMO
BACKGROUND: Metastatic phaeochromocytomas and paragangliomas (MPPGs) are orphan diseases. Up to 50% of MPPGs are associated with germline pathogenic variants of the SDHB gene. These tumours and many non-familial MPPGs exhibit a phenotype that is characterised by abnormal angiogenesis. We aimed to assess the activity and safety of cabozantinib, an antiangiogenic multi-tyrosine kinase inhibitor, in patients with MPPGs. METHODS: The Natalie Trial is a single-arm, phase 2 clinical trial being conducted at The University of Texas MD Anderson Cancer Center (Houston, TX, USA). Patients aged 18 years or older with histologically confirmed, progressive, and unresectable MPPGs, with an Eastern Cooperative Oncology Group performance status of 0-2, were treated with oral cabozantinib 60 mg/day. The primary endpoint was the investigator-assessed overall response rate per the Response Evaluation Criteria in Solid Tumours version 1.1 criteria. All outcomes were assessed in all evaluable participants who received any amount of study treatment. The trial is registered with ClinicalTrials.gov (NCT02302833) and is active but not recruiting. FINDINGS: From March 10, 2015, to May 11, 2021, 17 patients (13 male participants and four female participants) were enrolled. The median follow-up was 25 months (IQR 18-49). The overall response rate was 25·0% (95% CI 7·3-52·4; four of 16 patients). Seven grade 3 adverse events were reported in six patients, including single cases of hand-and-foot syndrome, hypertension, rectal fistula, QT prolongation, and asymptomatic hypomagnesaemia, and two cases of asymptomatic elevations of amylase and lipase. There were no grade 4 adverse events and no patient died on-study. INTERPRETATION: Cabozantinib shows promising activity in patients with MPPGs. FUNDING: Team NAT Foundation, Margaret Cazalot, and Clarence P Cazalot.
Assuntos
Neoplasias das Glândulas Suprarrenais , Anilidas , Paraganglioma , Feocromocitoma , Piridinas , Humanos , Piridinas/uso terapêutico , Piridinas/efeitos adversos , Feminino , Masculino , Pessoa de Meia-Idade , Anilidas/uso terapêutico , Anilidas/efeitos adversos , Feocromocitoma/tratamento farmacológico , Feocromocitoma/patologia , Feocromocitoma/genética , Paraganglioma/tratamento farmacológico , Paraganglioma/patologia , Adulto , Neoplasias das Glândulas Suprarrenais/tratamento farmacológico , Neoplasias das Glândulas Suprarrenais/secundário , Idoso , Inibidores de Proteínas Quinases/uso terapêutico , Inibidores de Proteínas Quinases/efeitos adversosAssuntos
Neoplasias das Glândulas Suprarrenais , Melanoma , Neoplasias Cutâneas , Humanos , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico , Melanoma/secundário , Melanoma/diagnóstico por imagem , Neoplasias Cutâneas/secundário , Neoplasias Cutâneas/patologiaRESUMO
ABSTRACT: Prostate carcinoma (PC) is the second most common malignant tumor in males globally. The metastatic spread of PC usually involves the pelvic and abdominal lymph nodes and the skeletal system. Cutaneous metastases are exceedingly uncommon and typically manifest themselves late in the disease course, considered as ominous sign with limited treatment options and a poor prognosis. We describe a patient wherein 68 Ga-PSMA-11 PET/CT detected multiple uncommon metastatic sites in the cutaneous region of the scrotum, penis, and thigh, as well as in the subcutaneous region of anterior abdominal wall, and in bilateral adrenal glands. These findings served as a theranostic tool for selecting 177 Lu-PSMA-617 treatment for these extremely rare metastatic sites.
Assuntos
Neoplasias das Glândulas Suprarrenais , Isótopos de Gálio , Radioisótopos de Gálio , Lutécio , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Neoplasias da Próstata , Neoplasias Cutâneas , Humanos , Masculino , Neoplasias da Próstata/diagnóstico por imagem , Neoplasias da Próstata/patologia , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/patologia , Compostos Heterocíclicos com 1 Anel/uso terapêutico , Ácido Edético/análogos & derivados , Progressão da Doença , Radioisótopos/uso terapêutico , Dipeptídeos/uso terapêutico , Idoso , Oligopeptídeos , Tela Subcutânea/diagnóstico por imagem , Tela Subcutânea/patologia , Medicina de PrecisãoRESUMO
The purpose of this paper is to summarize 3 methods for treating adrenal metastases with stereotactic body radiation therapy. This article is not meant to provide consensus guidelines but rather to present 4 practical examples of treatment techniques using different treatment platforms from 3 institutions.
Assuntos
Neoplasias das Glândulas Suprarrenais , Radiocirurgia , Humanos , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/radioterapia , Radiocirurgia/métodosRESUMO
BACKGROUND: The decision for resection of adrenal metastasis from colorectal cancers remain controversial and there is no proposed standard treatment. The aim of the article is to review the available literature on outcomes and complications rates following adrenalectomy for adrenal metastasis from colorectal cancer. METHODS: Relevant papers were identified through electronic databases. Data was extracted independently by two authors on a Microsoft Excel spreadsheet up to June 2023. RESULTS: A total of 55 studies were included in the final analysis (145 cases). A large proportion of patients had an uneventful postoperative recovery following surgical adrenalectomy. The mean length of follow up ranged from 2 months to 9.5 years. A total of 33 (22.8%) patients were alive and well with no evidence of local or systemic recurrence; 2 (1.4%) patients had recurrence in the bed of adrenalectomy; 2 (1.4%) patients were alive with recurrence in the contralateral adrenal gland; 4 (2.8%) patients were alive with extra-adrenal metastasis, and 7 (4.8%) patients were alive and well with no comments regarding local and systemic recurrence. Post-operative mortality following adrenalectomy was uncommon: 1 patient died due to systemic sepsis following anastomotic leak. 17.2% of patients died due to disease progression. CONCLUSION: If complete resection can be achieved, surgical adrenalectomy in the surgically fit patient should be strongly considered, especially in patients with solitary adrenal metastasis which may translate into survival benefits and potential surgical cure.
Assuntos
Neoplasias das Glândulas Suprarrenais , Adrenalectomia , Neoplasias Colorretais , Humanos , Adrenalectomia/métodos , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/cirurgia , Neoplasias das Glândulas Suprarrenais/mortalidade , Neoplasias Colorretais/patologia , Neoplasias Colorretais/cirurgia , Complicações Pós-Operatórias/epidemiologia , Resultado do Tratamento , Recidiva Local de NeoplasiaRESUMO
OBJECTIVE: The existing prediction models for metastasis in pheochromocytomas/paragangliomas (PPGLs) showed high heterogeneity in different centers. Therefore, this study aimed to establish new prediction models integrating multiple variables based on different algorithms. DESIGN AND METHODS: Data of patients with PPGLs undergoing surgical resection at the Peking Union Medical College Hospital from 2007 to 2022 were collected retrospectively. Patients were randomly divided into the training and testing sets in a ratio of 7:3. Subsequently, decision trees, random forest, and logistic models were constructed for metastasis prediction with the training set and Cox models for metastasis-free survival (MFS) prediction with the total population. Additionally, Ki-67 index and tumor size were transformed into categorical variables for adjusting models. The testing set was used to assess the discrimination and calibration of models and the optimal models were visualized as nomograms. Clinical characteristics and MFS were compared between patients with and without risk factors. RESULTS: A total of 198 patients with 59 cases of metastasis were included and classified into the training set (n = 138) and testing set (n = 60). Among all models, the logistic regression model showed the best discrimination for metastasis prediction with an AUC of 0.891 (95% CI, 0.793-0.990), integrating SDHB germline mutations [OR: 96.72 (95% CI, 16.61-940.79)], S-100 (-) [OR: 11.22 (95% CI, 3.04-58.51)], ATRX (-) [OR: 8.42 (95% CI, 2.73-29.24)] and Ki-67 ≥ 3% [OR: 7.98 (95% CI, 2.27-32.24)] evaluated through immunohistochemistry (IHC), and tumor size ≥ 5 cm [OR: 4.59 (95% CI, 1.34-19.13)]. The multivariate Cox model including the above risk factors also showed a high C-index of 0.860 (95% CI, 0.810-0.911) in predicting MFS after surgery. Furthermore, patients with the above risk factors showed a significantly poorer MFS (P ≤ 0.001). CONCLUSIONS: Models established in this study provided alternative and reliable tools for clinicians to predict PPGLs patients' metastasis and MFS. More importantly, this study revealed for the first time that IHC of ATRX could act as an independent predictor of metastasis in PPGLs.
Assuntos
Neoplasias das Glândulas Suprarrenais , Paraganglioma , Feocromocitoma , Humanos , Feocromocitoma/patologia , Feminino , Masculino , Paraganglioma/patologia , Paraganglioma/cirurgia , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/secundário , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto , Prognóstico , Nomogramas , Metástase Neoplásica , Biomarcadores Tumorais/metabolismo , Biomarcadores Tumorais/análise , Seguimentos , Fatores de RiscoRESUMO
PURPOSE: Data regarding treatment options and their efficacy for metastatic paragangliomas (mPPGL) is limited. This study aims to report a single center experience in treating mPPGL, comparing the efficacy and safety of various treatment approaches. METHODS: Retrospective analysis of patients with mPPGL treated at an Endocrinology Department of a cancer institute between January 2000 and October 2022. RESULTS: We analyzed 25 patients with mPPGL, 8 pheochromocytomas and 20 paragangliomas (12% multifocal), followed for a median of 9 [4; 14] years. Surgical approach, aimed at the primary tumor or at debulking of metastases, was the only treatment achieving complete response: 87% in primary tumor and 87.5% with debulking of metastases. These were long-lasting results with a duration of 69 (23.8; 136.8) months in primary tumor removal and 35.1 (15.3; 41) months in metastases debulking. As for other therapeutic approaches, such as radioactive isotopes, tyrosine kinase inhibitors, chemotherapy and external beam radiotherapy, the main outcome was stable disease, with few partial responses. At the last follow-up, 66% of the patients were alive, 15.4% were in remission and 84.6% had stable disease. Median overall survival was 14 years. The 5-year and 10-year survival rates from primary tumor diagnosis were 77.9% and 66.9% respectively, and from metastasis diagnosis were 67.4% and 55.6%, respectively. CONCLUSION: This is the only European single center analysis addressing outcomes of different therapies in mPGL. The results support surgery as a first-line treatment, being the only approach that may achieve complete response with satisfactory and long-lasting results.
Assuntos
Neoplasias das Glândulas Suprarrenais , Paraganglioma , Humanos , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , Masculino , Paraganglioma/terapia , Paraganglioma/patologia , Adulto , Neoplasias das Glândulas Suprarrenais/terapia , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/mortalidade , Idoso , Resultado do Tratamento , Adulto Jovem , Metástase Neoplásica , Feocromocitoma/patologia , Feocromocitoma/terapia , Feocromocitoma/secundário , Feocromocitoma/mortalidadeRESUMO
A 72-year-old man was referred to our urology department due to a giant adrenal tumor detected by computed tomography( CT). Endocrine screening showed that cortisol, renin, aldosterone, adrenaline, and noradrenaline levels were all normal, and there was no evidence of adrenal hyperfunction. The adrenal tumor was so large that we suspected malignancy. Contrast-enhanced CT of the abdomen was performed for qualitative diagnostic purposes, and showed wall thickening of the sigmoid colon extending for approximately 6 cm. Lower gastrointestinal endoscopy was performed and revealed a full circumferential type 2 tumor in the sigmoid colon. Biopsy results showed intermediate differentiated ductal adenocarcinoma. Tumor markers were as follows: CEA 23.1 ng/mL, CA19-9 962 U/mL. The adrenal tumor was suspected of being malignant due to its size, but imaging examinations did not lead to a diagnosis of primary or metastatic disease. There were no tumors other than those in the sigmoid colon and adrenal glands. Since complete resection was deemed possible, sigmoid colon resection and combined left adrenalectomy were performed for both a diagnosis and treatment. A histopathological examination revealed that the histology of the adrenal tumor resembled that of colorectal cancer, leading to a diagnosis of left adrenal metastasis from sigmoid colon cancer.
Assuntos
Neoplasias das Glândulas Suprarrenais , Neoplasias do Colo Sigmoide , Masculino , Humanos , Idoso , Neoplasias do Colo Sigmoide/patologia , Colo Sigmoide/patologia , Biomarcadores Tumorais , Neoplasias das Glândulas Suprarrenais/cirurgia , Neoplasias das Glândulas Suprarrenais/secundário , BiópsiaRESUMO
Presentamos el caso de un paciente varón de 67 años diagnosticado de adenocarcinoma de pulmón en estadio IV con dolor irruptivo oncológico (DIO) de difícil control. Durante el transcurso de la enfermedad el paciente necesitó varios ingresos por complicaciones, siendo la más común el mal control del DIO. Tras varios cambios en la pauta analgésica (rotación de opioides, cambios en tratamientos coadyuvantes y vías de administración), se consiguió buen control del dolor a pesar de progresión de la enfermedad de base. Finalmente, el paciente precisó ingreso hospitalario ante deterioro del estado general y claudicación familiar. Ante mal pronóstico, el equipo de Cuidados Paliativos y los familiares decidieron instaurar tratamiento con medidas de confort. De la relevancia del caso clínico que aportamos se concluye que es necesaria la existencia de equipos multidisciplinares formados y entrenados en este ámbito para un buen manejo terapéutico y una mejor calidad de vida en los pacientes
We present the case of a 67-year-old male with stage IV adenocarcinoma lung cancer who presented difficult control of breakthrough cancer pain. During the course of the disease, the patient had to be hospitalized several times due to complications. The most common complication was breakthrough cancer pain. After many changes in the analgesic treatment (opioid rotation, changes in coadjuvant drugs and routes of administration), good control of analgesic pain was achieved despite the progression of this underlying disease. Finally, the patient required being hospitalized due to deterioration of the general condition and family claudication. Faced with a por prognosis, the Palliative Care and Oncology team decided to establish treatment with comfort measures. We can conclude that the existence of multidisciplinary trained team is necessary for good therapeutic management and a better quality of life in patients
Assuntos
Humanos , Masculino , Idoso , Adenocarcinoma de Pulmão/patologia , Neoplasias Pulmonares/patologia , Dor do Câncer/tratamento farmacológico , Dor Irruptiva/tratamento farmacológico , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Adenocarcinoma de Pulmão/diagnóstico por imagem , Neoplasias Pulmonares/diagnóstico por imagemRESUMO
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